Abstract
The term “syndrome” is derived from the Greek, meaning “to run together.” By defi nition, therefore, syndromes are diverse. The term “syndrome” implies that a condition is defi ned by its evident features and not by a single underlying etiologic principle. Congenital syndromes are often characterized by differences in connective tissue structure and neurological control that affect the spine. However, there are some general principles which govern the management of spinal deformities in children. This chapter will highlight these principles and then discuss in detail several syndromes which present with early onset scoliosis: Marfan, Loeys–Dietz, Shprintzen-Goldberg, Ehlers–Danlos, Prader–Willi, Rett and Down syndromes.
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References
Akbarnia BA, Marks DS, Boachie-Adjei O et al (2005) Dual growing rod technique for the treatment of progressive earlyonset scoliosis. Spine 30:S46–S57
Akpinar S, Gogus A, Talu U et al (2003) Surgical management of the spinal deformity in Ehlers–Danlos syndrome type VI. Eur Spine J 12:135–140
Caird M, Wills B, Dormans JP (2006) Down Syndrome: the role of the orthopaedic surgeon. J Am Acad Orthop Surg 14(11):611
D’Astous JL, Sanders JO (2007) Casting and traction treatment methods for scoliosis. Orthop Clin North Am 38(4):477–484, v. Review
De Paepe A, Devereux RB, Dietz HC, et al (1996) Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet. 62(4):417-26.
Faber P, Craig WL, Duncan JL et al (2007) The successful use of recombinant factor IIa in a patient with vascular-type Ehlers–Danlos syndrome. Acta Anaesthesiol Scand 51:1277–1279
Hutchinson DT, Bassett GS (1990) Superior mesenteric artery syndrome in pediatric orthopaedic patients. Clin Orthop Relat Res 250:250–257
Jones KB, Erkula G, Sponseller PD et al (2002) Spine deformity correction in Marfan syndrome. Spine 27:2003–2012
Kosaki K, Takahashi D, Udaka T et al (2005) Molecular pathology of Shprintzen–Goldberg syndrome. Am J Med Genet A 140:104–105
Loeys BL, Chen J, Neptune ER et al (2005) A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in GFBR1 or TGFBR2. Nature 37:275–281
Loeys BL, Schwarze U, Holm T et al (2006) Aneurysm syndromes caused by mutations in the TGF-β receptor. NEJM 355:788–798
Mik G, Gholve PA, Scher DM et al (2008) Down syndrome: orthopaedic issues. Curr Opin Pediatr 20(1):30–36
Nabai T, Obata K (2006) Growth hormone therapy and scoliosis in patients with Prader–Willi syndrome. Am J Med Genet A. 140(15):1623–1627
Percy AK (2008) Rett syndrome: recent research progress. J Child Neurol 23:543–549
Robinson PN, Neumann LM, Demuth S et al (2005) Shprintzen–Goldberg syndrome: fourteen new patients and a clinical analysis. Am J Med Genet A 135:251–262
van Steensel MA, van Geel M, Parren LJ et al (2008) Shprintzen-Goldberg syndrome associated with a novel missense mutation in TGFBR2. Exp Dermatol 17: 362–365
Acknowledgements
The authors would like to thank Dr. Marc Asher for his contributions of several radiographs in this chapter.
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© 2011 Springer-Verlag Berlin Heidelberg
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Sponseller, P.D., Yang, J. (2011). Syndromic Spinal Deformities in the Growing Child. In: Akbarnia, B.A., Yazici, M., Thompson, G.H. (eds) The Growing Spine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-85207-0_16
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DOI: https://doi.org/10.1007/978-3-540-85207-0_16
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